Ulnar Sided Cleft Hand

I have previously posted on cleft hand with a number of discussions.  More to come on this topic in the future as well.  However, I wanted to share a less common type of cleft hand, the ulnar cleft hand. Dr Tonkin from Sydney Australia has written about this unusual condition in the Journal of Hand Surgery.  In this case series, he reports on 3 children and describes ulnar cleft hand and reconstructive options.  Often the pinky needs to be re- aligned and stabilized.  The cleft can also be narrowed although, as with the more typical cleft hand, is not necessarily a function improvement.  It can be, however, an important appearance issue.

I have recently cared for a delightful family with adopted children from China- one has bilateral ulnar cleft hand.  As makes sense with adopted children, the family is taking a watch and see approach rather than jumping into surgery.  I think this is great.  Obviously, we want to really understand the child's function before even considering surgery.  And, to this family, the appearance not such an important factor.

In ulnar cleft hand, there is usually a good thumb and at least one good finger.  That is the case below with this ulnar cleft hand.  However, in Dr Tonkin's article, some kids had four digits and some had 5.

Notice the really deep cleft on the pinky side of the hand.  This is the case for both hands.  Note also the great thumb- index web space.   There is good alignment of the thumb.  The child has excellent pinch and large object grasp.  The pinky is not particularly useful on either side with the ulnar cleft hand.

Ulnar cleft hand, bilateral.

Ulnar cleft hand, left.  See deep cleft.

Right ulnar cleft hand.

x-ray of ulnar cleft hand.

x-ray of ulnar cleft hand, right side.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

The Extra Thumb

An extra thumb, or radial polydactyly, can represent a difficult problem in congenital hand surgery.  The difficulty is that each child with an extra thumb is somewhat different.  There are certainly patterns (as we see with the Flatt Classification system) but not every thumb fits this classification system.  Zuidam and all (under the leadership of Steven Hovius in Rotterdam, The Netherlands) added to our understanding of radial polydactyly with these additional thoughts on the Topic.

The goals for reconstruction of the extra thumb are well accepted- a stable, straight thumb that functions well and looks good.  Patients don't like crooked thumbs (and nor do we) and no one likes thumbs that are not stable either (see our Publication). A thumb without stability is weak for pinch.  The surgeon's job is more difficult when the joints are not straight and the bones are not straight.  These situations require a more complex reconstruction of bone, joint, tendon, and muscle insertion.  Here is one such case of a Type IV extra thumb- which means the duplication is at the level of MCP joint with two proximal and distal phalanges (as you will see below).   In this case we had to narrow the metacarpal bone, reconstruction the ligament, and reconstruct the tendon insertion but did not have to cut and re- align the bones.

Radial polydactyly, type IV.  Not the deviation of both thumbs.

Radial polydactyly, type IV.  Not the deviation of both thumbs.  Palm view.

Radial polydactyly, type IV.  Not the deviation of both thumbs.  X- ray.

The outside thumb, the radial thumb, is removed to allow the best thumb (which is generally but always the inside thumb).  

Planned incision for radial polydactyly surgery.

Planned incision for radial polydactyly surgery.

Surgery allows straightening and stabilization to a now straight thumb.

The green sutures indicate where the ligament and the tendon were reconstructed for radial polydactyly.

After radial polydactyly surgery.

After radial polydactyly surgery.  Note that we avoid straight skin incisions as they can tighten over time.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Thumb Deformity after Polydactyly Reconstruction

Radial polydactyly, an extra thumb, can be more challenging to treat than other types of extra digits.   The surgeon cannot simply remove the extra thumb as ligaments need to be recreated and often bones and tendons need to be re- aligned as well.  Nonetheless, if the surgeon can create a stable, well- aligned thumb, the patient will do well both functionally and from an appearance standpoint (i.e., it will look good).  The other challenge is addressing the size of the thumb- often the thumbs are smaller than the normal thumb on the other hand.  Again, surgery can often help the size of the thumb but does not make it exactly the same as the normal hand (but it does not need to be an exact sized thumb).  The goal is a straight and stable thumb- as we have shown in the scientific literature.

If a thumb is not straight after radial polydactyly reconstruction, the patient may have trouble with pinch activities and may not like the way the thumb looks.  The options to treat the thumb are limited and include:
- Addressing any soft tissue problems (i.e., scar bands)
- Cutting and re- aligning crooked bones
- Making joints stiff (fusion).

This is one example of a child with a crooked thumb years after her initial surgery for an extra thumb.  She has trouble using the thumb in school and does not like the way it looks (it is very noticeable to her friends).   The deformity is from 1) a scar band and 2) bony instability at the joint.  Both issues need to be corrected in order to provide a satisfactory outcome.  An excellent result would be expected with surgery.

Deformity after radial polydactyly reconstruction

Another view of deformity of the thumb after radial polydactyly reconstruction.

Notable scar band which is contributing to the abnormal position of the thumb after radial polydactyly reconstruction.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu